Osteopetrosis is a rare hereditary bone disorder presenting with variable clinical features and is characterized by an increase in bone density and reduction of marrow spaces that result from a defect in the function of osteoclasts and, consequently, a decrease in bone turnover. This disease is generally divided into three types: severe infantile malignant autosomal recessive, intermediate mild autosomal recessive, and benign autosomal dominant. The prognosis of the first two types is very poor and is characterized by an early onset, usually within the first decade of life, and early death. The benign-type is characterized by a later onset and a longer life span. Ten percent of osteopetrosis cases develop osteomyelitis that usually involves the mandible. The osteomyelitis is generally caused by tooth extraction or pulpal necrosis. The leading cause of the increased rate of infection is thought to be a lack of adequate bone vasculature. Treatment of osteomyelitis secondary to osteopetrosis is controversial. Treatment regimens include high-dose systemic antibiotics coupled with thorough debridement of necrotic bone and primary closure of soft tissues, if possible. Hyperbaric oxygen has been used for the treatment of chronic osteomyelitis.